Observational Study
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jan 7, 2015; 21(1): 318-325
Published online Jan 7, 2015. doi: 10.3748/wjg.v21.i1.318
Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis
Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, David Shoseyov, Malena Cohen-Cymberknoh, Joseph Rivlin, Ori Efrati
Moran Lavie, Tzipora Manovitz, Daphna Vilozni, Sarina Levy-Mendelovich, Ifat Sarouk, Ilana Weintraubv, Ori Efrati, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children’s Hospital, Sheba Medical Center, Tel Hashomer 52621, Israel
David Shoseyov, Malena Cohen-Cymberknoh, Cystic Fibrosis Center and Pediatric Pulmonary Unit, Hadassah Hebrew University Medical Center, Jerusalem 91120, Israel
Joseph Rivlin, Cystic Fibrosis Center, Carmel Medical Center, Haifa 34362, Israel
Author contributions: Lavie M, Vilozni D and Efrati O designed the study, analyzed the data and wrote the manuscript; Manovitz T, Levy-Mendelovich S and Sarouk I collected the medical data and performed the statistical analysis; Weintraubv I, Shoseyov D, Cohen-Cymberknoh M and Rivlin J provided the medical data, analyzed the data and were also involved in the writing and editing of the manuscript.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Moran Lavie, Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children’s Hospital, Sheba Medical Center, Derech Sheba 2, Tel Hashomer 52621, Israel. moran.lavie@sheba.health.gov.il
Telephone: +972-3-530-2884 Fax: +972-3-534-5914
Received: June 16, 2014
Peer-review started: June 20, 2014
First decision: July 21, 2014
Revised: August 27, 2014
Accepted: September 29, 2014
Article in press: September 30, 2014
Published online: January 7, 2015

AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome (DIOS) in Israeli cystic fibrosis (CF) patients.

METHODS: This is a multi-center, comparative, retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012. Patients diagnosed with DIOS were defined as the study group. The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan. For the control group, CF patients with no DIOS were matched to the patients in the study group for age, sex, and cystic fibrosis transmembrane conductance regulator (CFTR) mutations. For both groups, the collected data included age, sex, CFTR genotype, weight, height, and body mass index. Clinical data included respiratory function tests in the last five years prior to the study, respiratory function test immediately before and after the DIOS event, number of hospitalizations, sputum culture results, and CF-related conditions diagnosed according to the CF clinical practice guidelines. In the study group, data on the DIOS treatment and tendency for DIOS recurrence were also analyzed.

RESULTS: The medical charts for a total of 350 CF patients were reviewed. Of the 350 CF patients, 26 (7.4%) were diagnosed with DIOS. The control group included 31 CF patients with no DIOS diagnosis. The mean follow-up period was 21.6 ± 8.2 years. The total of DIOS episodes in the follow-up period was 60. The distribution of DIOS episodes was as follows: 6/26 (23.1%) study patients had one episode of DIOS in their lifetime, 7/26 (26.9%) had two episodes, 7/26 (26.9%) had three episodes, and 6/26 (23.1%) had four or more episodes. Compared to the control group, DIOS patients had a significantly higher incidence of meconium ileus in the past (65.4% vs 0%, respectively, P < 0.02), more Aspergillus spp. colonization (34.6% vs 3.2%, respectively, P < 0.02), and a higher number of hospitalizations due to respiratory exacerbations (8.6 vs 6.2 mean total hospitalizations per follow-up period, respectively, P < 0.02). No other significant differences were found between the control and study groups. The conservative treatment of DIOS, which mainly includes hydration and stool softeners, was successful in 82% of the episodes. The survival rate was similar for both groups.

CONCLUSION: CF patients with DIOS suffer from recurrent hospitalizations and airway pathogen acquisition. Although recurrence of DIOS is common, conservative treatment is successful in most patients.

Keywords: Distal intestinal obstruction syndrome, Cystic fibrosis, Meconium ileus, Treatment, Recurrence

Core tip: Distal intestinal obstruction syndrome (DIOS) is a gastrointestinal complication of cystic fibrosis (CF). This study reviews the long-term follow-up of DIOS across two decades in Israeli CF patients. The DIOS patients were more prone to meconium ileus, Aspergillus spp. airway colonization and frequent hospitalizations then the DIOS-negative CF patients. DIOS recurrence was observed in 77% of patients. Conservative treatment was successful in 82% of the episodes, which makes it the treatment of choice for DIOS management. The survival rate was similar to the CF patients without DIOS.